The objective of this study was to assist clinicians in the diagnosis of the occipital neuralgia syndrome by describing its clinical characteristics. Bibliographies and clinical descriptions of occipital neuralgia syndrome were identified through a review of literature published between 1966 and 1993. A prospective case series was performed by the authors in a university emergency department during a 1-year period. Patients with unilateral aching pain of the head, coupled with pain in the distribution of the occipital nerve, Tinel's sign, and relief of pain after local anesthetic injection, were included. Patients rated pain relief on a 10-point scale. Twelve patients met the criteria for occipital neuralgia and were included in the study. All patients reported at least 80% decrease of pain after injection, and 42% had complete relief. Clinical features, other than headache, that were common in patients included tinnitus in 33%; scalp paresthesia, 33%; nausea, 42%; dizziness, 50%; and visual disturbances, 67%. Occipital neuralgia is a benign extracranial cause of headache, and it may be confused with other more serious headache syndromes. Recognition depends on an understanding of the symptoms along with a careful history and physical examination. Local anesthetic injections produce significant relief of the headaches and can aid in the diagnosis of the syndrome.